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Epidemiology
RarediseaseincidenceintheUS19/million/yearandprevalenceof19-75/100,000
Rareinchildren Peakbetweenage35-65 Morewomenaffected Familyh/ootherautoimmunediseases Ethnicbackgroundinfluencesurvivalanddiseasemanifesta2on.
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Clinicalfeatures
Raynauds Typicalskinchanges Esophagealandsmallboweldysfunc2on Inters22allungdisease Pulmonaryhypertension Renalcrises
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WhatisRaynaudsPhenomenon
Reversibleskincolorchanges:Whitetobluetored
Duetovasospasm Inducedbycoldofemo2on
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Raynauds:Pallorphase
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Raynauds:cyano2cphase
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CausesofRaynauds
Occlusivearterialdisease Rheuma2cdiseases:Scleroderma,CREST,MCTD,SLE,RA,
Myosi2s
Repe22vevascularinjuryHyperviscosity:Polycythemia,Cryoglobulinemia
Thoracicoutletsyndrome
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Typicalskinchanges
Tightthickskin,peakednosePursedmouth
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Saltandpepperpigmenta2on
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Microstomia
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Esophagealdysmo2lity:heartburn,and
refluxsymptoms
DilatedesophagusonEsophagogram
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ShortnessofbreathinScleroderma
DD,1-Inters22allungdisease:CXR,PFT,HRCTchest
-Pulmonaryhypertension:Echocardiogram,rightsided
cardiaccatheteriza2on
3-Cardiomyopathy,Heartfailure,4-Pericardialeffusion
5-Renalcrises
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Inters22alLungdisease
Majorcauseofmorbidly
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Renalcrisesandhypertensionin
scleroderma
Majorcomplica2on Earlyindiseasefirstfewyears Acuteonsethypertension HighRenin Renalimpairment Microangiopathichemoly2canemia,ThrombocytopeniaRenalfailurecanbereversibleifBPtreated
DrugofchoiceAngiotensinconver2ngenzymeinhibitor
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CREST
Calcinosis RaynaudsEsophagealdysmo2lity
Sclerodactyly Telangiectasias
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Calcinosis
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Sclerodactyly
Skinchanges,andflexioncontactures
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Telangiectasias
Dilatedcapillariesseenovertheface
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Diagnosis
Clinicalfeatures ANAposi2ve90%An2topoisomerase1an2bodyposi2ve(scl-70)30%indiffuse
An2centromerean2bodyposi2veinCRESTandlimitedscleroderma
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Treatment
Skin:noeffec2vetreatment,60%improvewith2me
Treathypertensionearlyandaggressivetopreventrenaldamageandotherhypertensioncomplica2ons
CalciumchannelblockersmayhelpRaynauds TreatILDandpulmonaryhypertension Avoidsteroids(mayprovokerenalcomplica2ons)
